• Chest · Sep 2022

    Multicenter Study

    Comparison of the Lung Clearance Index in Preschool Children with Primary Ciliary Dyskinesia and Cystic Fibrosis.

    • Jobst F Roehmel, Friederike J Doerfler, Cordula Koerner-Rettberg, Folke Brinkmann, Anne Schlegtendal, Martin Wetzke, Isa Rudolf, Simone Helms, Joerg Große-Onnebrink, Yin Yu, Thomas Nuesslein, Irena Wojsyk-Banaszak, Sebastian Becker, Olaf Eickmeier, Olaf Sommerburg, Heymut Omran, Mirjam Stahl, and Marcus A Mall.
    • Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Berlin, Germany.
    • Chest. 2022 Sep 1; 162 (3): 534542534-542.

    BackgroundPrevious studies showed that the lung clearance index (LCI) determined by multiple-breath washout (MBW) is sensitive to detecting early lung disease in preschool children with cystic fibrosis (CF). In preschool children with primary ciliary dyskinesia (PCD), data on the onset and severity of lung disease and on the sensitivity of the LCI as a noninvasive quantitative outcome measure remain limited.Research And Study QuestionIs MBW feasible and sensitive to detect ventilation inhomogeneity in preschool children with PCD?Study Design And MethodsThis was a prospective, cross-sectional, multicenter study and included preschoolers with PCD, preschoolers with CF, and healthy control (HC) participants. LCI was determined using nitrogen MBW and was compared among the three groups.ResultsLCI was determined in 27 children with PCD, 34 children with CF, and 30 HC participants (mean age, 4.8 years; range, 2.2-6.9 years). The LCI in preschool children with PCD was increased (median, 9.1; 95% CI, 8.6-10.3) compared with HC participants (median, 7.0; 95% CI, 6.7-7.1; P < .0001), but did not differ from preschool children with CF (median, 8.6; 95% CI, 8.4-9.7; P = .71). The feasibility in the PCD group was 93.1% and was similar to that in the CF group (91.9%) and in HC participants (85.7%; P = .55).InterpretationThis study demonstrated early onset of lung disease in preschool children with PCD and indicated that lung disease severity in PCD may be similar to that in CF during preschool years. These data support a need for early diagnostic monitoring and therapy and suggest the LCI as a noninvasive diagnostic tool and as a potential end point in clinical trials testing early interventions in children with PCD.Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

      Pubmed     Full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…